Scleroderma Systemic sclerosis
Conditions
Interventions
None listed
Sponsors
Radboud Universitair Medisch Centrum
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: - Age >=18 - Diagnosis of SSc according to the 2013 ACR-EULAR classification criteria - Diagnosed with pulmonary hypertension WHO group 1 (PAH) and (in combination with)/or WHO group 2 (PH due to LHD) according to ESC[6]/ERS guidelines. - Written informed consent
Exclusion criteria
Exclusion criteria: - Severe lung diseases (severe interstitial lung disease with a forced vital capacity
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| In this study we will explore parametric mapping to characterize the myocardium. Native T1 mapping is able to detect local and diffuse necrosis and fibrosis, whereas T2 mapping detects the extent of edema, which is used to assess activity of (subclinical) myocardial inflammation. Stress and rest perfusion CMR using adenosine will be used to assess focal perfusion defects and exclude relevant epicardial coronary stenosis, and additional T1-mapping will assess global myocardial (microvascular) perfusion. The presence and extent of myocardial inflammation, fibrosis and myocardial perfusion will be compared with the control group of IPAH patients. Additionally, the relation between disease severity and clinical status will be explored, and compared with the traditional late gadolinium enhanced images for the detection of focal disease. The established ShMOLLI (Shortened Modified Look-Locker Inversion Recovery) sequence will be used for T1 mapping. Although T1-relaxation using ShMolli is a reproducible and consistent sequence to distinguish normal from diseased myocardium, the normal value needs to be determined for each MRI-scanner, since the normal value is scanner (i.e. vendor, field strength, location) dependent. In order to validate the T1-relaxation times in healthy individuals in this CMR, 20 patients without myocardial disease scheduled for a non-myocardial CMR scan (e.g. for the follow-up of aortic dilatation) will be asked to participate. Patients will be asked to give written informed consent to acquire this additional sequence and to use the results for scientific purposes. | — |
Secondary
| Measure | Time frame |
|---|---|
| To compare SSc-patients with PH and IPAH-patients in regards to features on electrocardiogram (such as right axis deviation, right ventricular strain pattern, right atrial dilatation), and echocardiography parameters (estimated pulmonary arterial pressures by tricuspid valve- and pulmonary valve regurgitation velocity, right atrial area, right ventricle (RV) fractional area change, TAPSE, global longitudinal RV strain, the RV- and left ventricle (LV)- end diastolic and systolic volume, the LV ejection fraction and diastolic function). Besides high-sensitive troponin-T, NT-proBNP and CRP, differences in blood result analyses will include characterization of T-cells subset and pro-inflammatory and pro-fibrotic mediators in peripheral blood mononuclear cells. | — |
Countries
Netherlands
Outcome results
None listed